Ataxia


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    This guideline has been adapted for statewide use with the support of the Victorian Paediatric Clinical Network

  • See also:

    Acute Poisoning 
    Head injury 
    Hypoglycaemia guideline 
    Metabolic disorders

    Background to condition:

    • Ataxia results from inco-ordination of muscle movements, manifested as lack of balance with a staggering gait, difficulty sitting, unsteady movements or clumsiness.
    • In most children it is a benign condition. 
    • Assessment focuses on excluding serious and treatable causes including CNS infection and mass lesions.
    • May result from neurological disease, metabolic disease or other systemic disorders. 
    • Some conditions present with an unsteady gait due to weakness (pseudo ataxia), and a frightened or sick child may also appear ataxic

    Classification & causes

    Acute ( <72 hours duration, previously well child) Episodic (recurrent ataxia) Chronic
    Post infectious* 
    Toxins*
    Tumours*
    Trauma
    Metabolic
    Infections
    Vascular (Stroke)
    Immune inc ADEM
    Conversion disorder 
    Toxin ingestion  
    Basilar artery migraine
    Seizure disorder
    Metabolic 
    Brain tumours
    Hydrocephalus
    Metabolic
    Nutritional
    Congenital malformations
    Hereditary ataxias

    *Most common causes

    More information:

    Acute cerebellar ataxia

    • Most common cause of acute ataxia in children
    • Most commonly 2 - 7 years of age
    • Post-infectious autoimmune process (most commonly varicella; also mycoplasma, EBV, HHV6, Parvovirus B19, enteroviruses)
      • usually 5-10 days after prodromal illness, with or without exanthem
      • may occur after varicella disease or immunisation
    •  may occur after varicella disease or immunisation
    •  Acute onset of gait ataxia, dysarthria, nystagmus,
      • symmetrical
      • other signs less pronounced
    • Ataxia maximal at onset; usually improving within days, full recovery over 10 - 21 days (>90% by 2 months)

    Ingestion of drugs/toxins/complementary medicines  - see Poisoning guidelines 

    All cause impaired consciousness.

    Ask specifically about: 

    • phenytoin, benzodiazepine, antihistamines
    • ethanol, ethylene glycol, isopropanol
    • essential oils
    • complementary medicines

    Tumours

    • Brain tumours
      • usually present with normal conscious level
      • signs of raised ICP
      • usually gradual onset, may present acutely due to haemorrhage into tumour.
    • Neuroblastoma
      • paraneoplastic encephalopathy
      • progressive ataxia; myoclonus, opsoclonus.

    Acute Demyelinating Encephalomyelitis (ADEM)

    • Demyelinating disorder of the CNS, which is monophasic but multifocal in nature associated with encephalopathy.
    • Febrile illness 1- 4 wks before onset of symptoms
    • Lethargy/irritability to coma, associated with multiple neurological deficits inc pyramidal signs, optic neuritis, hemiplegia, ataxia
    • Dx clinical suspicion. LP shows pleocytosis and increased protein. MRI shows multiple areas of demyelination in brain and spine,
    • Rx: methylprednisolone, IVIG.

    Assessment

    Assess for presence or absence of associated neurologic abnormalities which suggest life threatening or treatable conditions.

     Any child with

    • an altered conscious state,
    • focal neurology,
    • meningism
    • posterior column loss,
    • signs of raised intracranial pressure
    • or weakness suggestive of pseudo ataxia

    should NOT be managed as Acute Cerebellar Ataxia.

    History

    • Time course (acute < 72hours)
    • Antecedents
      •  Recent viral illness (varicella, HHV6, EBV, parvovirus B19)
      • Rash
      • Head trauma
      • Labyrinthitis (primary or secondary to otitis media)
    • Cerebellar signs
    • Diplopia
    • Difficulty sitting
    • Slurred speech 
    • Other neurological symptoms
      • headache
      • behavioural change
      • altered conscious state
    • Family History
      • Metabolic disease
      • Hereditary ataxia
    • Drugs/toxins 

    Examination

    o   Conscious state, orientation.

    o   Thorough neurological examination is mandatory

    • Features suggestive of Acute Cerebellar Ataxia
      • cerebellar signs
      • Gait abnormalities- staggering or wide based
      • A negative Rhomberg test
      • Preservation of vibration and joint position sense
    • Features suggestive of Pseudo ataxia
      • Weakness
      • Absent reflexes (consider Guillain Barre Syndrome)
      • Mixed upper motor neurone and lower motor neurone lesions suggestive of ADEM or Multiple Sclerosis.
    • Localising signs are NOT suggestive of Acute Cerebellar Ataxia

    o   Vertigo (labyrinthitis, vestibular neuronitis) - Hallpike manoeuvre, ENT.

    o   Exclude signs of infection

    • Fever
    • Vomiting
    • Meningism 

    Investigations

    If there is a clear history of Acute Cerebellar Ataxia with preceding viral prodrome, then no investigations may be necessary, however close follow up will be required. If ataxia does not follow the expected time course or if suspect other causes thenconsider

    Imaging
    • CT identifies space occupying lesions, trauma, haemorrhage
    Bloods
    • Blood gas (electrolytes, blood glucose)
    Other
    • Anticonvulsant level, ethanol/ethylene glycol
    • FBE
    • LFT
    • Metabolic screening
    • Lumbar puncture after neuroimaging (do not delay treatment)

     
    Management

    Those with acute cerebellar ataxia post-varicella/viral illness can be discharged following senior review, and reviewed by a general paediatrician in 1-2 weeks. However those children who have unexplained ataxia require admission for investigation.

    Consider consultation with local paediatric team: 

    • Any child presenting with Ataxia

    When to consider transfer to tertiary centre: 

    • Localising signs
    • Raised ICP
    • History not typical of Acute Cerebellar Ataxia
    • Child requiring care beyond the comfort level of the hospital.   

      For emergency advice and paediatric or neonatal ICU transfers, call the Paediatric Infant Perinatal Emergency Retrieval (PIPER) Service: 1300 137 650.

    Follow up:

    • Patients assessed to have Acute Cerebellar Ataxia should be reviewed by their local paediatric team in 1-2 weeks. 

    Last updated September 2012